RESUMO
Health-related quality of life (HRQoL) is an important outcome from the perspective of boys with haemophilia and their parents. Few studies have captured the HRQoL of boys with haemophilia in developing countries. This article reports on the cross-cultural adaptation of the Canadian Haemophilia Outcomes - Kids Life Assessment Tool (CHO-KLAT) for use in São Paulo, Brazil. The CHO-KLAT(2.0) was translated into Portuguese, and then translated back into English. The original English and back-translation versions were compared by a group of three clinicians, whose first language was Portuguese. The resulting Portuguese version was assessed through a series of cognitive debriefing interviews with children and their parents. This process identified concepts that were not clear and revised items to ensure appropriate understanding through an iterative process. The initial back-translation was not discrepant from the original English version. We made changes to 66% of the CHO-KLAT(2.0) items based on clinical expert review and 26% of the items based on cognitive debriefings. In addition, two new items were added to the final Portuguese version to reflect the local cultural context. The final result had good face validity. This process was found to be extremely valuable in ensuring the items were accurately interpreted by the boys/parents in São Paulo Brazil. The results suggest that professional translators, clinical experts and cognitive debriefing are all required to achieve a culturally appropriate instrument. The Portuguese CHO-KLAT(2.0) is well understood by Sao Paulo boys/parents. The next step will be to test its validity and reliability locally.
Assuntos
Hemofilia A/psicologia , Hemofilia B/psicologia , Qualidade de Vida , Adolescente , Brasil , Criança , Humanos , Entrevistas como Assunto , Masculino , Apoio Social , Inquéritos e Questionários , TraduçãoRESUMO
Psychosocial outcomes are important in the perspective of boys with haemophilia. However, health-related quality of life (HRQoL) is based on self-report, and assumes adequate literacy. Yet, literacy is rarely assessed prior to data collection. This study sought to identify criteria that might indicate the level of literacy of children being recruited for clinical trials and to develop a simple method to prescreen those whose literacy was uncertain. We developed a brief screening tool in the form of two stories, at a grade 3 reading level, followed by comprehension questions. We applied the screening test to a sample of haemophilic boys between the ages of 7 and 13 years to assess their literacy. The data were analysed to determine the best criteria to use in identifying the ability to independently self-report for HRQoL studies. Twenty-four Brazilian boys (7.9-12.8) completed the testing. The results showed that 17 (70.8%) were literate (were able to both read and comprehend), and could complete a questionnaire without assistance. All boys over 11.0 years of age were sufficiently literate. Grade level was not found to be a helpful criterion. We recommend that all children under the age of 11.0 years be prescreened before providing self-reported HRQoL data. Those with limited literacy should be provided assistance to ensure comprehension of the questions. This is important to ensure high-quality data on HRQoL for future clinical trials.
Assuntos
Hemofilia A/psicologia , Hemofilia B/psicologia , Qualidade de Vida , Adolescente , Brasil , Criança , Escolaridade , Nível de Saúde , Humanos , Masculino , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: To evaluate thromboelastographic parameters and fibrinogen levels in women treated with transdermal 17beta estradiol. METHODS: 29 menopausal women with a history of venous thromboembolic disease were included. Nine patients composed the treatment (HT) group and 20 the control group. Coagulation was assessed by thromboelastography in samples of whole blood and platelet-poor plasma (PPP). The following thromboelastographic variables were measured: time for initial coagulation (R), blood clotting speed (K and the alpha angle), clot tensile strength (MA and G), global index of coagulation (CI) and fibrinolysis (LY30) and fibrinogen levels. RESULTS: There were no differences in the other parameters comparing both groups. Fibrinogen levels showed a 13.77 +/- 19.94% reduction in the HT group and a 5.51 +/- 8.09% increase in the control group after 6 months. CONCLUSIONS: Our data suggested that transdermal estrogen may not increase blood coagulability, but that it reduces fibrinogen levels in HT women.
Assuntos
Estradiol/administração & dosagem , Terapia de Reposição de Estrogênios , Estrogênios/administração & dosagem , Fibrinogênio/análise , Tromboembolia Venosa/sangue , Administração Cutânea , Adulto , Análise de Variância , Feminino , Humanos , Pessoa de Meia-Idade , Projetos Piloto , TromboelastografiaRESUMO
INTRODUÇÃO: A sinovite na hemofilia pode induzir às alterações nos receptores sensitivos e proprioceptivos articulares. Tal efeito favorece a redução de estabilidade e um desalinhamento corporal, podendo comprometer a funcionalidade das crianças acometidas. OBJETIVO: Avaliar o alinhamento postural dos membros inferiores e o desempenho funcional de crianças hemofílicas, relacionando-os com a incidência de hemartroses. MÉTODO: Foi avaliado um grupo experimental (GE) de 28 crianças hemofílicas e um grupo controle (GC) de 20 crianças sem hemofilia. O GE foi dividido de acordo com o grau de comprometimento articular: 16 crianças no grupo sem sinovite crônica (GSS) e 12 no grupo com sinovite crônica (GCS). Cada grupo foi subdividido por faixa etária: 3-4 anos, 5-6 anos, 7 anos. A avaliação consistiu da análise do alinhamento dos ângulos do joelho e do tornozelo através do programa Geometer Sketchpad. O desempenho das atividades funcionais foi avaliado através de testes modificados a partir do Exame Neurológico Evolutivo de Lefévre. RESULTADOS: Foi constatado que a média dos sangramentos dos hemofílicos aumenta com a idade (p< 0,001) e parece ser relacionada com o grau de comprometimento articular (GCS>GSS), (p< 0,059); o ângulo do joelho do GCS tende a um menor valor (semiflexão) quando comparado aos outros grupos; e há diferença significativa no percentual de desempenho nos testes entre grupos na faixa etária de 7 anos (GSS>GC e GSS>GCS), (p< 0,087). DISCUSSÃO E CONCLUSÃO: Crianças hemofílicas de 7 anos apresentaram menor desempenho funcional quando comparadas aos outros grupos. Este resultado ressalta a importância da fisioterapia precoce associada à terapêutica médica para evitar comprometimentos articulares severos.
Assuntos
Criança , Criança , Hemartrose , Hemofilia A , Extremidade Inferior , Manipulação Quiroprática , Especialidade de Fisioterapia , PosturaRESUMO
OBJECTIVES: To compare the effect of conjugated equine estrogens (CEE) and raloxifene on lipid profile and hemostasis. MATERIALS AND METHODS: A double-blind, randomized and parallel study was performed with 90 healthy postmenopausal women, aged 54 +/- 5 years, divided into three groups and submitted to daily therapy with either CEE 0.625 mg, raloxifene 60 mg or placebo for 4 months. The lipid profile, coagulation and fibrinolytic factors were analyzed. RESULTS: CEE increased the levels of high density lipoprotein cholesterol (HDL-C) from 49.0 to 56.8 mg/dl (p < 0.001), very low density lipoprotein cholesterol (VLDL-C) from 17.2 to 22.3 mg/dl (p < 0.001), and triglycerides from 86.0 to 111.7 mg/dl (p < 0.001), and decreased the levels of low density lipoprotein cholesterol (LDL-C) from 121.0 to 106.5 mg/dl (p < 0.001). The only significant effect of raloxifene was an increase in the levels of HDL-C from 46.0 to 47.8 mg/dl (p = 0.019). There was no significant reduction in LDL-C, from 115.5 to 110.2 mg/dl (p = 0.06), VLDL-C, from 21.7 to 20.0 mg/dl (p = 0.201), and triglycerides, from 108 to 100 mg/dl (p = 0.201). CEE decreased the levels of fibrinogen, from 370.5 to 326.8 g/l (p = 0.039) and the levels of antithrombin III, from 99.5 to 93.2% (p < 0.001). Raloxifene decreased the levels of fibrinogen, from 354.7 to 302.0 g/l (p = 0.009) and the levels of antithrombin III, from 102.4 to 98.5% (p = 0.039). CEE increased levels of protein C from 103.7 to 115.3 mg/l (p < 0.001) and raloxifene did not change the levels of protein C (107.9 to 105.1 mg/l; p = 0.158). CEE decreased the antigen levels of tissue plasminogen activator (t-PA) from 8.8 to 6.8 U/ml (p < 0.001), and of plasminogen activator inhibitor (PAI-1) from 30.8 to 21.6 U/ml (p < 0.010), whereas raloxifene had no significant effect on either t-PA, from 9.6 to 9.2 U/ml (p = 0.235) or PAI-1 antigen levels, from 32.1 to 30.4 U/ml (p = 0.538). CONCLUSION Both CEE and raloxifene exert significant effects on the lipid and coagulation profile. CEE had a more significant effect on fibrinolysis than raloxifene. These effects may have a significant impact on the cardiovascular risk that needs to be confirmed in larger studies.
Assuntos
Antagonistas de Estrogênios/farmacologia , Terapia de Reposição de Estrogênios/métodos , Estrogênios Conjugados (USP)/farmacologia , Estrogênios/agonistas , Cloridrato de Raloxifeno/farmacologia , Moduladores Seletivos de Receptor Estrogênico/farmacologia , Adulto , Idoso , Análise de Variância , Coagulação Sanguínea/efeitos dos fármacos , Fatores de Coagulação Sanguínea/análise , Fatores de Coagulação Sanguínea/efeitos dos fármacos , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/prevenção & controle , Método Duplo-Cego , Antagonistas de Estrogênios/administração & dosagem , Estrogênios Conjugados (USP)/administração & dosagem , Feminino , Fibrinólise/efeitos dos fármacos , Humanos , Lipídeos/sangue , Pessoa de Meia-Idade , Pós-Menopausa , Cloridrato de Raloxifeno/administração & dosagem , Moduladores Seletivos de Receptor Estrogênico/administração & dosagem , Resultado do TratamentoRESUMO
The presence of antiphospholipid (aPL) antibodies and antiphospholipid syndrome (APS) was researched in 57 children and adolescents with systemic lupus erythematosus (SLE). The frequency of aPL antibodies was 75.4% (anticardiolipin 70.2% and lupus anticoagulant 29.1%). The positivity for these antibodies fluctuated during the course of the disease. No association was found between aPL antibodies and clinical or laboratory manifestations or the autoantibodies studied, nor with the activity or gravity of the SLE. APS was diagnosed in 14% of the cases (eight patients), on average three years after the diagnosis of SLE. Four patients had arterial thrombosis (stroke, three; transient ischaemic attack, one; amaurosis fugax, two; renal, one), one presented with deep vein thrombosis (DVT) and three had involvement of small calibre vessels (osteonecrosis, two; transverse myelitis, one). Recurrences were observed in three of the eight cases (37.5%), with a mean interval of 13 months between the events. The presence of APS was associated with haemolytic anaemia, leukopenia, thrombocytopenia, coagulation abnormalities, ischaemic cerebrovascular accidents, amaurosis fugax, osteonecrosis and interstitial pneumonitis. A negative association was observed between APS and the presence of anti-Ro antibodies.
Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , Lúpus Eritematoso Sistêmico/complicações , Adolescente , Adulto , Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/imunologia , Doenças do Sistema Nervoso Central/complicações , Criança , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Pneumopatias/complicações , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/imunologia , Osteonecrose/complicações , Trombose/complicaçõesRESUMO
Aluminum (Al3+) overload is frequently associated with lipid peroxidation and neurological disorders. Aluminum accumulation is also reported to be related to renal impairment, anemia and other clinical complications in hemodialysis patients. The aim of the present study was to determine the degree of lipid peroxidation, platelet aggregation and serum aluminum in patients receiving regular hemodialytic treatment. The level of plasma lipid peroxidation was evaluated on the basis of thiobarbituric acid reactive substances (TBARS). Mean platelet peroxidation in patients undergoing hemodialysis was significantly higher than in normal controls (2.7 +/- 0.03 vs. 1.8 +/- 0.06 nmol/l, P<0.05). Platelet aggregation and serum aluminum levels were determined by a turbidimetric method and atomic absorption spectrophotometry, respectively. Serum aluminum was significantly higher in patients than in normal controls (44.5 +/- 29 vs. 10.8 +/- 2.5 microg/l, P<0.05). Human blood platelets were stimulated with collagen (2.2 microg/ml), adenosine diphosphate (6 microM) and epinephrine (6 microM) and showed reduced function with the three agonists utilized. No correlation between aluminum levels and platelet aggregation or between aluminum and peroxidation was observed in hemodialyzed patients.
Assuntos
Alumínio/sangue , Falência Renal Crônica/sangue , Peroxidação de Lipídeos , Agregação Plaquetária/efeitos dos fármacos , Adolescente , Adulto , Idoso , Alumínio/farmacologia , Feminino , Humanos , Peroxidação de Lipídeos/efeitos dos fármacos , Peróxidos Lipídicos/sangue , Masculino , Pessoa de Meia-Idade , Diálise Renal/efeitos adversos , Substâncias Reativas com Ácido Tiobarbitúrico/análiseRESUMO
Aluminum (Al3+) overload is frequently associated with lipid peroxidation and neurological disorders. Aluminum accumulation is also reported to be related to renal impairment, anemia and other clinical complications in hemodialysis patients. The aim of the present study was to determine the degree of lipid peroxidation, platelet aggregation and serum aluminum in patients receiving regular hemodialytic treatment. The level of plasma lipid peroxidation was evaluated on the basis of thiobarbituric acid reactive substances (TBARS). Mean platelet peroxidation in patients undergoing hemodialysis was significantly higher than in normal controls (2.7 ± 0.03 vs 1.8 ± 0.06 nmol/l, P<0.05). Platelet aggregation and serum aluminum levels were determined by a turbidimetric method and atomic absorption spectrophotometry, respectively. Serum aluminum was significantly higher in patients than in normal controls (44.5 ± 29 vs 10.8 ± 2.5 æg/l, P<0.05). Human blood platelets were stimulated with collagen (2.2 æg/ml), adenosine diphosphate (6 æM) and epinephrine (6 æM) and showed reduced function with the three agonists utilized. No correlation between aluminum levels and platelet aggregation or between aluminum and peroxidation was observed in hemodialyzed patients
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Alumínio , Falência Renal Crônica , Agregação Plaquetária , Alumínio , Estudos de Casos e Controles , Peroxidação de Lipídeos , Peróxidos Lipídicos , Diálise Renal , Substâncias Reativas com Ácido TiobarbitúricoRESUMO
Aluminum (Al3+) intoxication is thought to play a major role in the development of Alzheimer's disease and in certain pathologic manifestations arising from long-term hemodialysis. Although the metal does not present redox capacity, it can stimulate tissue lipid peroxidation in animal models. Furthermore, in vitro studies have revealed that the fluoroaluminate complex induces diacylglycerol formation, 43-kDa protein phosphorylation and aggregation. Based on these observations, we postulated that Al(3+) -induced blood platelet aggregation was mediated by lipid peroxidation. Using chemiluminescence (CL) of luminol as an index of total lipid peroxidation capacity, we established a correlation between lipid peroxidation capacity and platelet aggregation. Al3+ (20-100 microM) stimulated CL production by human blood platelets as well as their aggregation. Incubation of the platelets with the antioxidants nor-dihydroguaiaretic acid (NDGA) (100 microM) and n-propyl gallate (NPG) (100 microM), inhibitors of the lipoxygenase pathway, completely prevented CL and platelet aggregation. Acetyl salicylic acid (ASA) (100 microM), an inhibitor of the cyclooxygenase pathway, was a weaker inhibitor of both events. These findings suggest that Al3+ stimulates lipid peroxidation and the lipoxygenase pathway in human blood platelets thereby causing their aggregation.
Assuntos
Alumínio/farmacologia , Peroxidação de Lipídeos/efeitos dos fármacos , Agregação Plaquetária/efeitos dos fármacos , Adulto , Alumínio/análise , Guaiacol/análogos & derivados , Guaiacol/farmacologia , Humanos , L-Lactato Desidrogenase/análise , Lignanas/farmacologia , Medições Luminescentes , Galato de Propila/farmacologia , Ristocetina/farmacologia , Salicilatos/farmacologiaRESUMO
Platelet aggregation was studied in a patient with familial hypercholesterolemia immediately after apheresis selective for low-density lipoprotein (LDL), a lipid-lowering procedure. This treatment reduced plasmatic levels of total and LDL-cholesterol, apo B, and triglyceride. Increased platelet aggregation was reduced immediately after the apheresis in whole blood as well as in platelet-rich plasma. However, aggregation in washed platelets remained unchanged after LDL-apheresis. In conclusion, in this patient reduction of LDL-cholesterol improved platelet function in the very short term.